Hainaut P, Lavenne E, Magy J M, Lebacq E G
Clin Rheumatol. 1986 Jan;5(1):96-101. doi: 10.1007/BF02030976.
We report the case of a young woman, with mixed connective tissue disease (MCTD), associated with disabling pulmonary hypertension and presence of the "lupus anticoagulant". The "lupus anticoagulant", an antibody directed against phospholipid components, was linked in our patient to extensive thrombophlebitis and premature labor. Raynaud's phenomenon progressed towards finger necrosis in spite of optimal vasodilating treatment. The part played by the "lupus anticoagulant" in pulmonary hypertension remains to be established. Both these complications responded to prednisolone therapy, but the improvement was limited and short-lived.
我们报告了一例年轻女性病例，患有混合性结缔组织病（MCTD），伴有致残性肺动脉高压且存在“狼疮抗凝物”。“狼疮抗凝物”是一种针对磷脂成分的抗体，在我们的患者中与广泛的血栓性静脉炎和早产有关。尽管进行了最佳的血管舒张治疗，雷诺现象仍发展为手指坏死。“狼疮抗凝物”在肺动脉高压中所起的作用仍有待确定。这两种并发症对泼尼松龙治疗均有反应，但改善有限且持续时间短。
